| Objective: To investigate the clinical and laboratory features of pemphigoid nodularis(PN),compare the clinical differences between nodular pemphigoid and bullous pemphigoid(BP),improve the understanding of the diagnosis and treatment of PN,reduce misdiagnosis and missed diagnosis,summarize the diagnostic process,and provide a reference for clinical decision-making.Methods: This study was divided into two parts: clinical case analysis and literature review.Part I,Clinical case analysis: The clinical data of 8 patients with pemphigoid nodularis and 182 patients with bullous pemphigoid who were diagnosed by the first diagnosis in the inpatient department of the First Hospital of China Medical University from September 2017 to September 2022 were collected for retrospective analysis,and two typical cases were reported;Part II,Literature review: By searching CNKI,VIP,wan-fang,Pub Med and other domestic and foreign databases,the cases of PN with pruritic nodules who were diagnosed by histopathological and immunological examination methods were collected and systematically analyzed from the general data,clinical features,pathological and immunopathological characteristics,immunological examination results,treatment and other data of the patients.Results:I.Clinical analysis1.General conditions: 8 cases of PN: 4 males and 4 females,with an average age of70.38 ± 7.07 years.182 cases of BP,84 males and 98 females,the male to female ratio was 0.86:1,with an average of 67.96 ± 11.55 years.2.the characteristics of lesion findings: the initial lesion had blisters: PN in 1 patient(1/8,2.5%)and BP in 62 patients(62/182,34.1%).Lesions were severe,PN in 6patients(6/8,75%)and BP in 112 patients(112/182,61.5%).3.diagnosed or misdiagnosed as at least one other type of skin disease: PN in 8patients(8/8,100%)and BP in 68 patients(68/182,37.4%).4.Duration of disease: 8 cases of PN: the longest 6 years,the shortest 4 months,with an average of 24.63 ± 21.02 months;182 cases of BP: the longest 5 years,the shortest5 days,with an average of 3.64 ± 1.06 months.There was a significant difference between PN and duration of BP(P = 0.026).5.Disease control time: 8 cases of PN: the longest 42 days,the shortest 14 days,with an average of 24 ± 7.92 days;182 cases of BP: the longest 53 days,the shortest 4 days,with an average of 13.87 ± 7.36 days.There was a significant difference in disease control time between PN and BP(Z =-2.462,P = 0.014).II.Literature review1.General conditions: 92 cases of PN: 42 males and 50 females,male to female ratio of 0.84:1,with an average of 63.16 ± 15.87 years.There were 27 patients without blisters,65 patients with blisters,and the mean age of patients without blisters was68.44 ± 16.51 years;the mean age of patients with blisters was 60.97 ± 15.05 years.There was a significant difference in the age of illness between patients without blisters and patients with blisters(Z =-2.599,P = 0.009).2.Clinical features: 81 cases(88.0%)had generalized skin lesions,83 cases(90.2%)involved the trunk,87 cases(94.6%)involved the extremities,18 cases(19.6%)involved the head,face and neck,8 cases(8.7%)involved the hands and feet,and only 1 case had oral mucosal involvement without blisters.They had been misdiagnosed: 35(35/92,38.0%),14(14/27,51.9%)without blisters,and 21(21/65,32.3%)with blisters.3.Histopathology: Of the 83 patients with skin biopsy data,50(50/83,60.2%)had subepidermal fissures(blisters),35(35/83,42.1%)had EOS sponge edema,42(42/83,51.9%)had superficial dermal EOS infiltration,45(45/83,55.6%)had epidermal hyperkeratosis and acanthosis.4.Immunological examination: positive number(rate): 90 cases underwent DIF: 85cases(85/90,94.4%),23 cases without blisters(23/27,85.2%),62 cases with blisters(62/63,98.4%);42 cases underwent IIF: 36 cases(36/42,85.7%),14 cases without blisters(14/17,82.6%),22 cases with blisters(22/25,88%);30 cases underwent salt fissure test: 28 cases(28/30,93.3%),9 cases without blisters(9/10,90.0%),19 cases with blisters(19/20,95.0%);34 cases underwent anti-BP180(ELISA): 27 cases(27/34,79.4%),10 cases without blisters(10/15,66.7%),17/19,89.5%);26 cases underwent anti-BP230(ELISA)46.2%),9(9/13,69.2%)had no blisters and 3(3/13,23.1%)had blisters;34 underwent anti-BP180 and/or BP230(ELISA): 30(30/34,88.2%)and 13(13/15,86.7%)had no blisters There were blisters in 17 patients(17/19,89.5%).There were significant differences in the positive rates of DIF detection and anti-BP230 antibody detection between the non-blister group and the blister group(P< 0.05).Conclusions: Patients with PN have long duration of symptoms(approximately 24.6to 44.7 months)before correct diagnosis.Itching symptoms in elderly patients are a common denominator in PN patients.Clinicians should perform skin biopsies and immunological analysis in elderly patients with unexplained or refractory chronic pruritus,erythema,plaques,papules,and nodules. |
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